As I sit quietly awaiting a CT scan of my diseased sinuses wearing a 24-hour holter monitor, a neck brace and a mask over my face to protect my weakened immune system in a waiting room full of strangers, I wonder, when did my life get so complicated? Just under ten years ago I was having the time of my life, working and going to school full-time while enjoying an active social life. I have to be totally honest here: I really, really miss those good ol’ days, before chronic illness struck out of the blue and overnight. Since 2007 I have endured countless doctor’s appointments, invasive tests, and probably had hundreds of tubes of blood drawn out of my body. Diagnosis after diagnosis has accumulated on my medical record, which has now become a dauntingly long and complicated list. I Just. Want. To. Be. Normal. Again.
In the midst of the worst sinus infection of my life I reflect upon these things. Two and a half months of antibiotics and on my third one now and we’re deciding whether or not it’s really time for surgery, which has been delayed for a few years due to a host of new and complex conditions arising. Sinus surgery has not exactly been top priority until now, because the antibiotics have stopped working. I am terrified at the prospect of surgery, but my facial pain is so severe and unrelenting that I am desperate for some relief. A whole year of steroids have wreaked havoc on my immune system and my body needs a break from fighting the giant sinus infection that is my life.
I’m certain many of you can relate to how I’m feeling. Frustrated, overwhelmed, angry, perplexed, scared and a bit sad that I’ve had to endure so many maladies over these past ten years while the majority of my peers have been living it up: traveling, partying and achieving major milestones. It was definitely not easy losing most of my twenties to chronic illness. 30 was the worst year yet due to getting hit by yet another scary and sudden illness called Guillain Barre Syndrome, which stole my ability to walk within three days time, and which I’m still not completely recovered from. I still have some residual nerve damage in my legs and probably my arms, but at least I can walk again and open a package of cheese for myself. So far 31 isn’t shaping up to be so grand either. My chronic sinusitis is clearly kicking my butt and with that I’ve had some frighteningly familiar neuromuscular symptoms return. I could scream from the sheer frustration of still being a reluctant passenger on the seemingly never-ending doctor merry-go-round.
But, if the words Albert Einstein so eloquently spoke are true, does opportunity really lie in the middle of difficulty? Perhaps I should be approaching this surgery optimistically, as the goal is to help me breathe better, which may end up improving many of my other symptoms as well. It’s all about shifting my perspective of the operation from fear and dread to optimism and opportunity.
I hope you, dear reader, have been doing well during my absence. It’s the beginning of a new year and I wish you improved health and happiness!
The past few months I've been stagnating in survival mode due to horrendous, persistent pressure headaches at the base of my head and upper neck. Throw in some charlie-horse muscle spasms on the sides and back of my skull and in my upper neck that are palpable to the touch and it's a recipe for intense pain, the kind that keeps me awake at night. When I finally do pass out from pain and exhaustion, the pain promptly awakens me again in a mere two to three hours. Subsisting on two to three hours of sleep per night is enough to render even the sanest of people completely mad. This lack of sleep, coupled with intense chronic pain really gets me down at times. I feel like a zombie most days and on my "good" days where the pain is bearable and I can attempt to distract myself from it, I get overzealous and overdo it because I never know how long the low-pain stretch will last and I feel the need to take advantage of those times whenever I can. However, on the low-pain days, all my body seems to want to do is catch up on sleep, but my mind is telling me I need to get out of the house and enjoy whatever I can before the headache becomes hellish again. The worst part of all this is, my doctors still can't seem to tell me with any certainty whether this head pain is a result of my 5mm chiari malformation or more due to my atlantoaxial instability (right lateral subluxation of C1 on C2). Or it could possibly be occipitoatlantoaxial instability (meaning my ligaments are so lax due to EDS that they're failing to hold my head on straight). But again, not even neurosurgeons can pinpoint with any certainty what exactly is causing the intense pain.
My best bet, no doubt, would probably be to travel to a place like The Chiari Institute of New York where they fix the instability and decompress the chiari in the same 6-hour procedure, taking a team approach with two surgeons present in the operating room, one to fuse the upper vertebrae and one to decompress the chiari. That means (hopefully) only having to go under the knife once and ensuring that my bobblehead is stabilized. However, New York might as well be Mount Everest to me right now as travel has become just about impossible lately. Even car rides in my trusty Aspen Vista cervical collar have become almost unbearable. Any movement really triggers and worsens my neck and head pain, which would suggest instability is likely the pain-causing culprit. This is not to say that my chiari is not causing me any problems. My balance has become really bad this past year or so. I used to be so graceful, so poised back in the olden days when I danced ballet. Now I am so clumsy I am tripping over my own feet, dropping things left and right and I have to concentrate really hard on basic tasks like walking or carrying something. I know that is not normal, not for me, not for anyone. I used to enjoy going on walks even after getting sick with POTS, but lately I fear that I look like a drunk person trying too hard to keep my balance and I wonder if people think I'm a drunk when I'm out in public...
I know that it's definitely time to do something. I've been told by my physical therapists, neurosurgeons and primary care physicians that unfortunately physical therapy is never going to remedy the problem, no matter how diligent I am with my exercises. My ligaments are too lax thanks to stupid EDS hypermobility. And while physical therapy can help to strengthen the surrounding supportive muscles and hopefully preserve the rest of my neck function and help provide stability to my neck's lower vertebrae (I currently have four bulging discs in my c-spine and some mild arthritis between all my vertebrae), the upper vertebrae are probably going to require a cervical fusion procedure to correct the instability. The prospect of a risky neurosurgery makes me sick to my stomach.
In terms of major surgery I've only had an emergency appendectomy, and fortunately I didn't have too much time to ruminate on the issue or be scared as I wasn't really given a choice. I do remember asking the on-call surgeon if I could just go home, research the procedure for a bit and then return the next morning for the surgery. He informed me that I could do that, but then he'd see me again in a day or two at most with a ruptured appendix and have to perform an even more painful surgery with a much longer hospital stay. So I consented to have a laporoscopic appendectomy that same night, and honestly, it was the best decision I ever made! I was in the hospital recovering for one day and then they released me the next night. Luckily the nurses and anesthesiologist were all very careful with me because of my dysautonomia and EDS. I was well-hydrated and there were no complications. I took some extra strength Tylenol at home and didn't even need the narcotic pain meds they prescribed. It hurt, sure, but in a few days the worst of the pain had subsided and I was able to get on with my life and start eating and drinking normally again.
I have a feeling that neurosurgery is a whole different ballgame though. I don't like the thought of anyone cutting into my neck and head. I just don't. It's icky. Necessary, but icky, and frankly very scary. There's too much important stuff up there. I have thought long and hard about what will happen to me if I don't have surgery though and the possibilities are also scary. I can't get in any more car accidents or sustain any type of major injury as it could easily be game over for me. Internal decapitation does not sound like a good way to go. So although it will be scary and painful, I have decided I need to stop being so afraid and proceed with surgery at the soonest opportunity. I do not want to live perpetually in pain and a neck brace. It would be nice to not have to wear this thing indefinitely. And even nicer to be out of pain. I can barely remember what it feels like to be pain-free but I know it would be amazing and I could accomplish so much more with my time and get so much more out of life.
Most importantly, if I get my health back I can devote 110% of my energy and focus to helping others who are still in pain and suffering from chronic, painful and misunderstood conditions like dysautonomia, EDS and chiari: the trifecta or "The Sara Syndrome" as the brilliant Dr. Rekate has coined it. Also, if you haven't seen it already, last week The Today Show featured a news segment about a young boy suffering from the debilitating effects of chiari malformation, dysautonomia and EDS. Please watch his story and share with your friends and family. This kind of national, mainstream media coverage is a huge deal for patients like us.
This presentation by Dr. Rekate really resonated with me. It captures the typical experience of patients suffering from dysautonomia, joint hypermobility, chiari malformation and craniocervical instability and the associated sensitivities and autoimmune problems we struggle with.
The most common form of Ehlers-Danlos syndrome (EDS type 3) occurs in an estimated 1 in 5,000 people, according to Medline Plus. It is a genetic disorder that affects connective tissues throughout the body. The pain of EDS varies from mild to extreme, but can become chronic over time and require lifelong physical therapy as well as surgical and pharmacological treatment. There are many types of EDS, which may have multiple comorbid conditions. For instance, some individuals with EDS type 3 also have a hindbrain herniation called Chiari malformation (CM). Some research suggests that having EDS may dispose people to developing an acquired form of Type I CM that can be difficult to manage appropriately even with advanced surgical intervention.
Genetics and Birth Defects While EDS develops before birth, CM in EDS patients can be acquired later in life as a result of the unstable connective tissues throughout the neck and spine pulling the brain downward. Cranio-cervical Instability (CCI) and Tethered Cord Syndrome (TCS) are features found regularly in EDS patients that may contribute to the occurrence or severity of the CM. EDS is considered an inherited disorder because it is caused by genetic mutations that affect collagen. Collagen is a building block of proteins that gives connective tissues their strength. According to the Lister Hill National Center for Biomedical Communication, the following gene mutations may lead to EDS:
· COL1A1 and COL1A2 · COL3A1 · COL5A1 and COL5A2 · ADAMTS2 · TNXB · PLOD1
Although CM may indeed have a genetic component in the congenital form, the only genetic link in the acquired form is the presence of connective tissue disorders like EDS. While considered an uncommon disorder, CM typically appears during childhood or early adulthood, although symptoms can surface at anytime and may be triggered or exacerbated by accidents, falls or blows to the head. During this stage of development, CM can become apparent because the skull cannot adequately contain the cerebellum and this crowding at the base of the skull can disrupt the essential flow of cerebral spinal fluid (CSF), as well as impinge important nerves and even disrupt blood flow. While CM is largely considered a congenital condition by most neurosurgeons, there is a growing body of evidence that it runs in families, just like EDS, and furthermore, that people with EDS may be predisposed to developing CM, although they were not necessarily born with the CM as a congenital abnormality.
Similarities and Differences in Pain Perhaps the biggest similarity between EDS and CM is chronic pain. People with EDS are often misconceived as being clumsy or as having imaginary pain because of the fact that they may look completely healthy on the outside. The reality is that the range of pain spans from mild to severe, but can occur anywhere along the body where connective tissues are present. If you suffer from EDS, you may bruise easily and have unusual scars after a wound heals. Your skin may be unusually stretchy, and you might have prominent, visible blood vessels. EDS is also known for causing joint pain and frequent joint dislocations and subluxations throughout the body, which can necessitate the need for joint bracing and careful physical therapy to retain independence and mobility.
CM causes several different types of chronic pain, including a severe characteristic headache at the base of the skull, as well as pain throughout the neck, spine, and throat. It can also trigger severe nerve pain throughout the body. Individuals afflicted with CM may experience serious neurological deficits, trouble swallowing, tinnitus, vision disturbances and balance disruption that can lead to trouble walking and/or clumsiness.
Does EDS Cause CM? Several studies suggest there is a complex connection between the two disorders that goes beyond the typical “cause and effect” association. According to the Hypermobility Syndromes Association, the causes of CM in a patient with EDS may be different than those in a patient without EDS. Furthermore, EDS patients seem to be more prone to Type 1 CM, which is the most common form of the disorder. The Association reports that a study of 2,813 Type 1 CM patients found that about 357 of them also had EDS symptoms.
More studies are needed to determine precisely how EDS causes CM. The only way to accurately determine the severity and cause of your condition/s is to seek appropriate testing and evaluation from a team of specialized medical professionals including neurologists, neurosurgeons and geneticists. EDS Type 3 can be diagnosed by a geneticist who administers a Beighton Scale Test while CM can be diagnosed by a neurologist or neurosurgeon through an MRI of the brain and cervical spine, preferably an upright MRI in those with suspected connective tissue disorders. Ideally, you should also seek out a doctor that specializes in chronic pain disorders to help manage the daily pain since medical testing isn’t the only assessment of pain disorders.
Kristeen Cherney is a freelance health and lifestyle writer who focuses on preventive measures for a better quality of life. Cherney holds a BA in Communication, and is currently finishing her MA in English.
Thanks so much to Mary over at A Body of Hope for nominating me for the One Lovely Blog Award! Here are seven facts about me:
1) I have a growing nail polish collection and recently started dabbling (quite literally!) in nail art. 2) My favorite season is fall. I love all the warm hues, crisp cool air and pumpkin spice everything at Trader Joe's! 3) Earlier this year I was diagnosed with Chiari malformation and craniocervical instability secondary to Ehlers-Danlos syndrome. A few more serious diagnoses to add to the list. I am trying my best to cope with these both physically and emotionally. 4) I am currently writing a book. So many readers have told me I should write a book since I started this blog years ago and I am finally taking their advice! 5) I have mild OCD tendencies, particularly when it comes to organization, cleanliness and germs. I have never been officially diagnosed but even as a small child cleaning my room put me at ease and everything had to be in its place. As a result, I had the neatest, prettiest pink room on the block! 6) Earlier this year I experienced a startling revelation that I am no longer as young and cool as I used to be...I am still listening to stuff I listened to as a kid in the 90's and 2000's, and I still think it's much better than this newfangled music kids these days listen to! ;) 7) I wear yoga pants just about everyday. Today marks the first time in two months I have put on a pair of jeans. Why? Because yoga pants are much more comfortable, and the type I wear provide subtle compression which helps with hypermobility!
I will be contacting my nominees via Facebook shortly! I look forward to reading your posts.
On the eve of a very important appointment with a very important neurosurgeon, I find myself brimming with anxiety over the occasion. You'd think that after seven years of health drama, doctor's appointments wouldn't even phase me anymore. But the reality is, sometimes they still do. Especially the important ones. So I figured what better way to spend my evening than to engage in a little blogtherapy and update you on what's been happening in my life so far this year and also explain why I haven't updated this blog in ten months!
Let's start off with some happy news! In February I got engaged! The proposal was magical and perhaps I should save that story for a separate post! We were both so thrilled that we started wedding planning almost immediately but then we both took a step back and realized that planning a huge wedding was starting to get really overwhelming (and really expensive!) really quickly. The more I thought about it the more I realized that a wedding is merely one day and that the marriage itself is what really matters. So we went from wanting a huge outdoor wedding and reception to now wanting a very small, private, low-stress ceremony and will probably skip having a formal reception altogether. It just doesn't make sense to exhaust all of our physical and financial resources on one single day when the time, money and energy expended could be better invested in creating our home together. I've basically stopped wedding planning for now and I'm so much more at ease. We've agreed upon the perfect small venue and I am going to wear a gorgeous gown. I also want a cool, Cake-Boss style cake. But other than that our wedding will be no-frills, no fuss.
In April we started fixing up my fiance's house to put on the market. Boy was that a job! It really tested our patience as things just kept going wrong and the house proved to be a lot more work than any of us expected! Several weekend work parties, one dead cat (long story!) and countless hours of manual labor later, the house is finally listed on the real estate market and being shown! Cross your fingers for us that it sells soon so we can move forward with the next chapter of our lives.
In June I finally had an upright MRI of my brain and cervical spine as ordered by my primary care doctor for the wonky discs in my neck that kept going out. The MRI results confirmed my biggest fears: that I do indeed have a Type 1 Chiari malformation as well as cervical instability secondary to Ehlers-Danlos syndrome. I always knew Chiari was a distinct possibility because of my EDS, but I figured since my POTS has gotten so much better over time that I surely I didn't have any more comorbid conditions to worry about. Well, I was wrong. My Chiari measures a mere 5mm but I am symptomatic with headaches, balance issues and tingling extremities and have been for over a year now. So far I have seen a local neurosurgeon, had a Skype consult with Dr. Rekate of The Chiari Institute (who was wonderful and a wealth of information!) and have one more consult with the head of neurosurgery at the local University tomorrow. Dr. Rekate told me he trusts this surgeon's skill so I feel a bit better in knowing that he's highly regarded by his peers. However my cervical instability is perhaps more of an issue than the Chiari itself so I need to make sure my local doctors take it seriously and address it accordingly.
In July and August, I ended up getting sent for several additional tests: an MRI of my thoracic spine, an MRI of my lumbar spine, cervical x-rays in flexion and extension, a 3D CT scan of my cervical spine, and a cine MRI of my brain/c-spine. Am I ever sick and tired of MRI's! The good news is that my MRI's showed NO evidence of a syrinx or a tethered cord. Tomorrow I will receive the results of the cine MRI from the neurosurgeon and boy am I nervous. The cine MRI is a specialized MRI that assesses the cerebral spinal fluid flow or extent of the obstruction from the Chiari. I am hoping he tells me I don't need surgery, or at least not urgently. The prospect of brain/spine surgery scares the bejeezus out of me. And I would really like to focus on things like getting married and finding our new home. A surgery would certainly disrupt my plans. But will have to see what he says tomorrow and take everything one step at a time.
Can't believe it's already October which also happens to be dysautonomia awareness month! This month I look forward to getting 3 teeth crowned (ugh!) and watching copious amounts of Gilmore Girls on Netflix. It's also my mom's 60th birthday which is kind of a big deal. Not quite sure how to commemorate the occasion but she has been a huge support to me my whole life and especially these past few months as I have suffered some pretty rough days health-wise and dealt with yet another life-altering diagnosis. Wish I could buy her a really cool, expensive gift! Or send her on a vacation somewhere tropical and relaxing!
As for November and December, we are hoping my fiance's house will finally sell so we won't have to maintain it over the winter and I am really really hoping to avoid neurosurgery. I should know more about that tomorrow. Until then, thanks for reading! I have really missed blogging and missed hearing from my lovely readers as well! Hope you are all doing well and enjoying improved health and wellness!
I am honored to have been nominated for a Liebster Award by one of my favorite new bloggers and fellow potsy Brittany at Fabulously Faint. Admittedly I don't know much about the award except for the following: The Liebster Award is used by bloggers who have 200 follows or less on bloglovin' to promote each other and to make more friends in the blogging community and attract more attention to their blog. Each nominee must first list 11 random facts about themselves and then answer the 11 questions proposed by the person who nominated them. Lastly choose 11 bloggers and ask them your own 11 questions.
11 Random Facts About Me! 1) I have Postural Orthostatic Tachycardia Syndrome, Ehlers Danlos Syndrome and Temporomandibular Joint Disorder. 2) I am obsessed with 90's pop culture nostalgia: toys, junk foods, music, television etc. 3) I adore the color pink and always have. Because some things never change. 4) My all time favorite book is The Great Gatsby. 5) I have perfectionistic tendencies and am very OCD about germs and cleanliness, though I have never been officially diagnosed. 6) I hold three bachelor's degrees including a degree in English Literature. 7) I loathe technology and hate trying to keep up with all the latest and greatest gadgets. I resisted the whole touchscreen smartphone craze for a LONG time. 8) I am a dog person. If there are 20 people in the room and there are any dogs in that room, the dogs will always come sit on my lap. This phenomenon has happened on many occasions. We get each other and speak the same language! 9) I used to produce magazine style shows and host talkshows for government television. 10) I have naturally curly hair that is extremely hard to tame. I prefer to wear it straight but everyone else prefers it curly. 11) I used to dance ballet, jazz and modern dance and still really miss it. Ballet was my favorite. Here are my questions courtesy of Brittany at Fabulously Faint: Question #1 If you could only eat one food for the rest of your life what would it be?
Fruit Tarts. Undoubtedly fruit tarts. Anyone who hasn't tried one of these fine delicacies absolutely needs to. I would happily eat them everyday for the rest of my life. Fruit, custard, chocolate. All in one pretty little package. What's not to love? Question #2 What's the most exciting/adventurous thing you have ever done? Although I'm about as far from an adrenaline junkie as you can get, I'd say the most exciting thing I've ever done was meeting my favorite band and having a drink with the lead guitarist a few months before I got sick. I could've died happy that night. Question #3 Describe yourself in three words. Creative, kind and funny. Question #4 Who inspires you? Chronic illness survivors! That includes a lot of strong people. And anyone who stands up for injusticeespecially in the face of adversity! It takes a lot of strength to do such a thing. There are too many people I admire to name just one! Question #5 What is your favorite piece of clothing? My pink peacoat. It personifies me! Question #6 If you could switch lives with someone for a day who would it be? Someone who is in perfect health and optimal physical condition. I would take advantage of being in a healthy body for a day, that's for sure! Question #7 If you could choose any career what would it be? An interior designer! It wouldn't even feel like work to me, I would likely enjoy every second of it! Question #8 What is your guilty pleasure? Reality TV. Shows like Keeping Up With The Kardashians and Dance Moms especially. Question #9 What is your dream vacation? Australia. I have always, always wanted to see the Sydney Opera house on New Year's Eve and to hold a koala bear! Question #10 Who are your style icons?
Anna Nalick. I was at her concert recently and we happened to be wearing the exact same ankle booties! Her style is unpretentious yet pretty and feminine. Timeless, not trendy. She always utilizes vintage or eclectic finds skillfully. She is not flashy but classy and refined and doesn't feel the need to dress trashy. Question #11 What is your biggest achievement? Being Valedictorian and voted most likely to succeed in my high school yearbook. It was flattering that my peers had such a favorable opinion of me and nice to be valued for my intellect. Especially these days now that POTS-induced brainfog has seemingly clouded my once sharp intelligence. If nothing else, it's comforting to know I was once a brainiac!
"...I was dancing again, every muscle effortlessly engaged, articulating in perfect synchronicity."
About a month ago, one of my best friends sent me an article that brought me to tears. While I'm sure her intention wasn't to make me cry, this article hit way too close to home. She sent it to me because she knew it would resonate within me like it did within her. We had both been dancers in our former pre-POTS lives. More specifically, ballerinas. Living a beautiful life doing what we loved and were perhaps born to do. We were introduced to each other through a mutual friend who also had POTS and recognized that we were destined to be besties. We bonded instantly over our similar past experiences and our current shared love of reality dance TV. While watching Breaking Pointe is no substitute for dancing ourselves, it certainly does help fill the void.
The night before I received the article in my Facebook inbox, I had had a dream that I was dancing again. It was haunting, but not a nightmare. It was a very vivid, beautiful dream that made me feel very much alive and self-actualized in those brief moments I that was dancing again, every muscle effortlessly engaged, articulating in perfect synchronicity. Unlike my current reality, I had complete control over my own body, and it felt good. Exhilarating even. I was in my old ballet class with my former instructor Miss Mary guiding my movements across the floor. Except I was my current self in present day, just in an alternate reality. A reality much more fulfilling than my own.
In my present reality, I am currently in physical therapy for a bum ankle. Or at least what I thought was a bum ankle that to my dismay actually turned out to be two bum ankles and a bum hip. It's no secret that years of ballet takes a toll on one's body. And perhaps even more of a toll when you're hypermobile and repeatedly encouraged by over-zealous instructors to relish in your perfect turn-out. While I haven't danced ballet for several years, apparently I still walk like a duck, my hips stuck in a perpetually turned-out state. My physical therapist also happens to be hypermobile and is good at correcting these issues. She uses pilates as a way to strengthen muscles and thereby control her own hypermobility. I did not realize how bad my ankles had gotten until she said that I need to learn how to walk all over again. Apparently I've been doing it wrong all these years.
As I raised into releve alongside a supportive countertop in physical therapy, I kept having flashbacks to the ballet barre and and all the hours that I had spent there, nearly deforming my own body from three years of age onward, as it was growing and developing into the body I have today. Going to ballet class was as routine as brushing my teeth in the morning. As the years of class went on, all our bodies grew to be the same shape and size, so that we were all nearly identical and could all wear the same size costume if need be. There was certainly not much physical diversity in ballet. Most of us were naturally hypermobile to some degree. If you weren't, you simply couldn't compete in the ballet world. Or, you had to work very, very hard at it. Those are the type of dancers I respect the most. The ones who have to work at it. Now that I'm aware of my hypermobility, I realize I possessed an unfair advantage in the ballet world.
I have been asked the following question several times by friends and family: if you could go back in time knowing what you know now, would you have still danced ballet? The answer is undoubtedly yes. Although it may have caused irreparable damage to my body, I was simply meant to do it. And there has always been a void since I stopped dancing. I consider myself a generally happy person, but there's always been something missing. And I know precisely what that something is.
Ballet was my outlet, my escape, my passion. It helped quell the obsessive compulsive tendencies I had hidden as a child. It also came so naturally, more naturally than sports or other athletic pursuits. I didn't have to work at it. It was effortless. The rest of my life has certainly not been effortless by comparison. Physical therapy is hard work. I am developing a new awareness of my body, and an appreciation for what I put it through. I am trying hard to correct bad habits that ballet helped me acquire over the years and push through the pain. I carry with me everyday now the physical reminders of having once been able to do what I loved. And to me, it's all been worth it. If I could still dance ballet today, I absolutely would.
No, it is NOT erectile dysfunction syndrome awareness month, nor is "EDS" an acronym for that! EDS stands for Ehlers-Danlos Syndrome and May marks a special month of awareness for this life-altering genetic connective tissue disorder which often results in extreme joint hypermobility and frequent dislocations among myriad other symptoms. I did not learn I had this condition until long after my POTS diagnosis. Many people with EDS also suffer from dysautonomia as a secondary condition. My POTS and TMJ are likely both linked to EDS as an underlying cause.
As a former ballet dancer I have always been very flexible. And that was a major attribute growing up. But never realized I had stretchy skin until an orthopedist pointed it out. And more importantly, I never realized that these seemingly harmless traits would predispose me to other serious health issues like POTS. I am very fortunate that my joints seem to be holding up okay so far (knock on wood!) and feel for all the EDS patients who have already had multiple joint replacements at my age or younger. EDS manifests differently in each patient although most of us share those hallmark traits of flexible skin and joints. Some people have very severe cases which can result in disability and even death, and others are plagued by milder cases that don't impose a major impact on daily life.
If you are extremely flexible and suspect you or someone you know may suffer from EDS, please talk to your doctor about it. And consider getting a referral for genetic testing. Diagnosis can lead to more comprehensive, tailored treatment and can help doctors and surgeons determine safer means of treatment. For instance, when I was struck with appendicitis and found myself in an emergency situation, I was able to inform the surgeon and anesthesiologist of my EDS before surgery. They took extra special care of me because of my EDS diagnosis, paying careful attention to the position of my jaw during surgery, as it is prone to dislocations. Knowledge is power. Take ownership of your health to help your healthcare team provide you with the best care possible!
Although I don't write a Christmas letter or anything of the sort, I figure my dear blog readers tune in from time to time throughout the year and are probably all too aware of the health goings-on in my life. So I will try my best to keep this brief and not too negative. It has been one heck of a year to say the least!
Let me start with the good. Certain aspects of my health have improved significantly. My resting heart rate is good! Normally anywhere between 56 and 66 beats per minute on any given day. And there have been many days where my standing heart rate hasn't been so bad either! (70's, 80's, 90's, I rarely reach the 100's anymore!) I am always thrilled to check my blood pressure monitor and see relatively normal numbers there too. It helps me breathe easier when I think about how those same numbers used to look a year ago. Can I say my POTS is gone? Well, no. There are days when my HR still spikes upon standing, I still get the occasional dizzy spell and brain fog seems to plague me on a daily basis. But when I compare my symptoms and my numbers to what they were 5 years ago the difference is dramatic. And there are even some days where I even feel somewhat normal.
In terms of chronic pain and limitations however, this has been the worst year of my life. The TMJD pain is unbearable half the time and it impacts my life severely. I have had to drastically modify my diet to soft foods exclusively and master the art of being quiet which has proven very difficult both emotionally and functionally. It is really tough to not be able to make phone calls to doctors or insurance companies. Not to mention not being able to speak to friends and family very often. I have had to shell out $1300 for a home ultrasound machine like the kind they used on me in Physical Therapy after my insurance company cut off physical therapy completely. It is about the only thing that brought me any real relief. I am currently going through the appeal process with my insurance company about covering the ultrasound machine as durable medical equipment for a chronic condition.
A local neurologist also thinks Botox injections would help quell the muscle spasms in my jaw and facial muscles, but insurance does not want to cover that either because it is not FDA approved for anything other than the treatment of migraines (which I am not having). Although my medical insurance does cover TMJD, they certainly don't seem to cover any of the treatments that have worked for me. So the battle continues. Sadly, this type of battle has become all to familiar to many of us. Having to fight for the basics all the while fighting our own battles against our ailing bodies. My wish for the new year is that less people will suffer and struggle with their insurance companies. That we will not have to fight as hard to receive adequate treatment and relief.
May my friends and readers experience improved health and relief from chronic conditions in 2013. Keep on fighting the good fight and never never never give up!
I have a huge aversion to vampires. I have not seen a single Twilight movie and have a feeling I'm not missing out on much. I don't buy into the whole "vampires are cool" craze. There are just too many darn vampires on TV these days. So I found myself surprised when I could not stop watching the UK version of Being Human. It came highly recommended by a fellow potsy with great taste in television, so I decided to give it a try. Aside from being permanently scarred for life by a few gory scenes, I actually gleaned quite a lot of good out of this show and found myself empathizing with the main characters' struggle to be "normal" humans.
As irony would have it the "monsters" and ghost are not the true evil characters on the show. It is the Catholic priest, the misguided professor and the sociopath fiancee (all real humans) who are perhaps the most evil and seem to lack any sort of profound moral compass. Although they make big mistakes, the non-humans are actually good-hearted and consistently try to help people in spite of their own issues.
Here is how I personally relate to each of the main characters:
Annie: I wouldn't be surprised if a lot of POTS and EDS patients easily relate to Annie the ghost. She spends most of her time at home as a quiet observer of the goings-on around her. She is invisible to almost everyone and cannot be heard either. She cannot really "live" her life as she is a ghost and had all her earthly dreams and goals ripped from under her when she was pushed down the stairs. Her afterlife lacked purpose and she is often struggling to find her place in the world. She is arguably the nicest character on the show and genuinely goodhearted and wholly undeserving of what happened to her. She also wears the same outfit in every episode. Comfy clothes and cute Ugg boots.
George: George is a good-hearted werewolf who wants desperately to lead a normal life. He has to hide his secret from friends, coworkers and even tried to hide it from lovers. Although I don't deem his problem to be quite as severe as the vampire or the ghost's considering he only has to deal with spontaneously transforming into a werewolf one night a month during the full moon, it is still a huge disruption to his life. I relate to George because I too transform into a very unpleasant person during the first few days of my period and therefore I try to lay low and avoid people during that time so I don't say something I'll later regret.
Mitchell: Although I find it hard to relate to Mitchell much at all, a part of me does feel sorry for him. Sympathy for a vampire? That doesn't sound like me at all, but it could have something to do with the fact that he is really, really, ridiculously good looking. Although he has some bad relapses, he tries hard to be a good vampire and not feed on humans anymore. Mitchell perhaps most strongly resembles the drug addict the show's writers had originally intended for his character. He also struggles to form meaningful and lasting relationships with the other characters except for George and Annie that is.
The first three seasons of Being Human (only 8 episodes each!) are currently available on Netflix. I highly recommend this show if you like sci-fi or drama. Be prepared to shut your eyes for a few scenes if you have a weak stomach like me. Other than that it's a great show that can teach you a lot about humanity, values, and perhaps most significantly, the struggle to maintain normalcy with an unwanted affliction. Many of us with chronic illness can relate.
I have grown increasingly intrigued by oral and injectible amino acid therapy ever since stumbling upon lessflexible.com. Amino acids are complicated and I don't claim to know the first thing about them, but I do wonder if they could help improve the strength of our stretchy connective tissues as they did in one young woman's experience. She claims she is less flexible since starting a carefully tailored and administered amino acid therapy regime with her doctor. I would love to be less flexible, as would most EDS sufferers. Amino acid therapy an area that I believe warrants further research in relationship to connective tissue disorders. I am happy to welcome guest blogger Martina from Gracewell Healthcare who graciously tackled the basics of amino acids.
Amino
acids consist of a mixture of carbon, hydrogen, oxygen and nitrogen.The human body
naturally stores twenty of these acids. However, another nine must be obtained
from food sources. It is absolutely essential that we have every single one of
the amino acids, as they each perform a special function. In general terms they
combine to form proteins, which are important for the building and maintenance
of muscles and processing of the brain. Amino acids have also been found to
play a role in weight reduction.
Where:
To Find Them
There
are a wide range of foods and drinks that are high in protein. Meats such as
pork and beef fall under this category and contain the complete variety of
amino acids. Less fattening alternatives such as fish and chicken are also
complete amino sources. Those individuals who refuse to consume meat or dairy
products can still maintain the protein levels by eating vegetables, nuts and
whole grains. Health experts advise the consumption of at least 60g of proteins
every single day. Some people may even opt to maintain the healthy diet with
supplements.
How:
To Consume/Recognise/Identify Them
In
scientific terms amino acids are formed from a central carbon atom, to which
the Carboxlyic acid, Hydrogen, Amino and variable 'R' groups are attached.
Amino acids can join together in the formation of dipeptides and polypeptides.
They have fairly complex names such as phenylalanine, which is known as a
provider of energy and tryptophan, which can combat sleep deprivation and
depression. As previously mentioned, the standard amino acids form part of our
natural DNA structure. The essential amino acids must be consumed as food or
drink.
When:
They Come Into Play/Their Function in the Human Body
A
high profile scientist by the name of Dr. Elson Haas has stated that around one
fifth of the human body is made up of proteins. They are a major constituent
part of the eyes, skin, muscle and brain. Without these proteins human beings
wouldn't exist. Apart from keeping us alive the proteins and amino acids that
they are made up from play a key role in the processing of messages from the
brain and generation of energy. People who are unable to maintain their amino
acid levels may experience depression, insomnia and extreme lethargy.
Why:
They Are Important
It
is essential that humans consume foods and drinks containing amino acids on a
daily basis. That's because the body uses them in a variety of means, including
the building of organ and muscle tissue and development of hormones such as
adrenaline. It is definitely worth taking supplements that include vital aminos
such as Threonine, which prevents the onset of illness and disease, and Leucine, which allows for the
processing of vitamins, minerals and protein. Anybody who fears that they may
not be consuming enough amino acids or requires assistance on the selection of
supplements is advised to see a medical expert.
Ironically, one of my best friends put this Rascal Flatts song on a mix CD for me on my 22nd birthday right before I got sick. Since then, the lyrics have taken on a whole new meaning. I relate to music much differently now than I did before I got sick. That's what life experience and struggle bring to the table. A deeper understanding and appreciation of language and lyrics. You feel like a candle in a hurricaneJust like a picture with a broken frameAlone and helpless, like you've lost your fightBut you'll be alright, you'll be alright ‘Cause when push comes to shoveYou taste what you're made ofYou might bend ‘til you break‘Cause it's all you can take On your knees you look upDecide you've had enoughYou get mad, you get strongWipe your hands, shake it offThen you stand, then you stand Life's like a novel with the end ripped outThe edge of a canyon with only one way downTake what you're given before it's goneAnd start holdin' on, keep holdin' on ‘Cause when push comes to shoveYou taste what you're made ofYou might bend ‘til you break‘Cause it's all you can take On your knees you look upDecide you've had enoughYou get mad, you get strongWipe your hands, shake it offThen you stand, yeah, then you stand Every time you get upAnd get back in the raceOne more small piece of youStarts to fall into place, yeah ‘Cause when push comes to shoveYou taste what you're made ofYou might bend ‘til you break‘Cause it's all you can take Yeah, then you stand
Do you ever wonder what might have been if you hadn't been struck by chronic illness at an early age? For some of us, the onset was gradual. But for others, like me, the onset was startlingly abrupt. One day I was dancing and hosting talkshows and the next I was down in bed barely able to move. Five years later, I have seen some significant improvement but my current self is still a far cry from my former self who could tackle multiple tasks on a daily basis without thinking twice about it.
Ever since I got sick, I have been haunted by my former self. Valedictorian voted "most likely to succeed," president of (nearly) every high school organization and college graduate with three degrees. I had the world at my fingertips it seemed and it was ripped away from me in a heartbeat the day I got sick. People from my past who don't know the whole story of what happened to me must assume that I've become a failure or a recluse, not living up to the outlandish expectations imposed on me by a small town and its overly-interested inhabitants.
While it's true that I care entirely too much of what others think of me, sometimes I can't help but feel sorry for myself. Maybe it's how the chronically ill are conditioned. People are either pitying us, or not believing us. There is very little empathy shown by others. Trying to observe my own situation objectively as if I were an outsider looking in on my own life, I must admit it's a pretty pathetic picture most of the time. Even older people in poor health pity my life, or shall I say existence, as a twentysomething. They know that when they were my age, or even in their thirties, forties, fifties, they were able to do as they pleased without any significant bodily restictions or limitations imposed on their lives. They traveled, raised families, played sports. I do none of those things. I am stuck in every sense of the word. Every now and then, I see a glimmer of my former, pre-illness self start to surface but then quickly fade when my physical symptoms kick in, reminding my current self that it cannot do what it used to, or what it had planned to do in the future.
What would my life look like today if I weren't sick? I ask myself that question all the time. I would likely be living in a high-rise minimalist penthouse in the city with a career that I enjoyed and comfortably afforded my lifestyle. I would have a vibrant social life filled with people my own age and dinners rarely eaten at home alone. I would barely sleep because I would be too busy enjoying life, but when I did sleep it would be restful, restorative sleep. I would shop every single day if I so chose, simply because I'd have the ability to remain upright for long periods. I would be building a house that I designed myself and planning for a future marriage, family, etc. I would take tons of road trips. And sometimes, I would drive for miles and miles and miles just because. I would go to Australia. Heck, maybe I'd live there for a bit. I would dance ballet again, or even teach it. I would live my own life and live it well without caring about what anyone else thought. I would never have doctors appointments to deal with. I would be happy.
I often wonder why those who lack ambition in life seem to never really get sick. Many people would be content to stay home day in and day out living a life of isolation with little stimulation. But not me. This type of existence is for the birds, certainly not for people like me. I can't pretend I am content with my situation. I am not there yet. I want things to change for the better in a big way. I want to be 100% my old self again. That girl had reasons to get up in the morning and push herself and her body let her do everything she wanted to. I want my body to cooperate with my mind's wishes like it used to. I want them to be in sync with each other so that I can be my complete self again instead of the sad ghost of my former self I have become.
As any ballet dancer knows, perfect turnout is considered paramount to being successful in the ballet world. Here is an example of picture perfect turnout:
Now, for many of us with EDS, this position comes naturally. It always did for me. As a child my ballet teacher was often impressed by my innate "perfect turnout" and would use me as an example to demonstrate for the rest of the class. A few of my peers were able to easily emulate this ideal turnout, and others could barely get their feet to point anywhere but straight forward. They were probably the "normal" ones in the bunch. Little did my teacher know that I was simply a genetic freak anomaly who didn't have to work to acquire this turnout at all. I loved ballet. It came pretty naturally and I was able to use my body as a vessel for self-expression. Had I have known that I may have been doing more harm than good to my body though, perhaps I wouldn't have let my teacher work me so hard. Perhaps I wouldn't have danced ballet at all actually. Everytime I watch Dance Moms and see Brooke Hyland engage in her picturesque contortions I can't help but wonder if she has EDS and want to warn her to slow down now because her body will thank her for it later. I worry that any child who exhibits extreme hypermobility may be at risk for extreme pain, suffering and even surgeries later in life. Especially if the hypermobility is encouraged by demanding teachers (like Abby Lee Miller!) or even parents who don't realize the potential consequences of overstretching their children.
Perhaps someday the medical population will realize that EDS is much more common than the literature recognizes and start testing for hypermobility routinely at physicals. They should definitely start screening for it at ballet schools, gymnastics gyms, etc. If nothing else, early screening may enable parents to help their kids protect their joints through good practices and bracing at an earlier age, thereby prolonging the life of a problematic joint. Instead it is often considered cool to share these party tricks with others. In fact, our culture values hypermobility on display. Everytime I watched street performer and self-described extreme contortionist hiphop dancer "Turf" do his thing on America's Got Talent I couldn't help but root for him. Not only was he a likable guy, it also seems likely that he will endure some medical problems down the road as a result of his dancing now. Disclaimer: It probably goes without saying, but just in case you're new to this blog I should mention that I am not a licensed physician and therefore not qualified to make medical diagnoses for any of the aforementioned individuals based on what I have seen them do on TV. They may or may not have ehlers-danlos syndrome.
